目的分析出血型烟雾病(MMD)的临床及影像学特征。方法回顾性分析20例出血型烟雾病的临床资料,观察其临床及影像学特点。结果本组患者主要集中在35~45岁年龄组,其中80%为女性。头颅CT扫描示脑室系统出血、脑实质出血及蛛网膜下腔出血;DSA表现为颈内动脉末端狭窄或闭塞,脑底烟雾状血管网、侧枝循环血管网以及动脉瘤形成。结论DSA检查是确诊烟雾病的主要方法,临床上对于无血管病变危险因素的脑出血患者均应常规行DSA检查。 Objective To analyze the clinical and imaging features of haemorrhagic moyamoya disease (MMD). Methods The clinical data of 20 cases of haemorrhagic moyamoya disease were retrospectively analyzed. The clinical and imaging features were observed. Results The patients were mainly in the age group of 35-45 years, of whom 80% were female. Head CT scan showed ventricular system hemorrhage, cerebral parenchymal hemorrhage and subarachnoid hemorrhage; DSA showed the end of stenosis or occlusion of the internal carotid artery, cerebellum smoked vascular network, collateral circulation vascular network and aneurysm formation. Conclusions DSA is the main method for the diagnosis of moyamoya disease. DSA is routinely performed in patients with cerebral hemorrhage who are risk factors for vascular disease.